The I-DSD Registry was conceived following the Chicago Consensus meeting in 2005 when the need for pooling standardised information in cross border registries was identified as a priority. With the help of a small project grant from the European Society of Paediatric Endocrinology Research Unit in 2006, a group that consisted of Faisal Ahmed (Glasgow), Silvano Bertelloni (Pisa), Sten Drop (Rotterdam), Olaf Hiort (Luebeck), Ieuan Hughes (Cambridge) and Richard Sinnott (Glasgow) developed an initial ESPE DSD Register.

The prototype ESPE DSD Register became the EuroDSD Registry when it was supported by EUFP7 between 2008 and 2011 as part of the EuroDSD project. By the end of the EuroDSD project, the Euro-DSD Registry was supporting several centres in countries beyond Europe and in 2011 it became the International DSD (I-DSD) Registry when it secured a 5-yr international partnership grant from the UK MRC. During this period, the I-DSD Registry was also including cases of CAH and to facilitate this further, the Registry developed a mirror site which used the same registry platform as I-DSD, the I-CAH Registry, and which was launched in 2014. Over the last few years, the I-DSD Registry has also included cases of Turner Syndrome and in 2020, the project also started work on developing another mirror site (I-TS) which could use the same registry platform as I-DSD and I-CAH.

After the end of the MRC support in 2017, the I-DSD and I-CAH Registries have been supported from funding from a wide range of sources that includes fees incurred by investigators for obtaining data for research, project grants from UK NIHR, the Chief Scientist Office of Scotland, income from the biennial symposium and unrestricted education grants from the pharmaceutical industry, including Diurnal and Neurocrine Biosciences. 

In 2021, the I-DSD/I-CAH/I-TS has a network that reaches 260 centres in 63 countries on all the continents. Of these, 115 centres from 40 countries use the registries and have entered over 5,500 cases where information can be shared for a range of purposes that have the ultimate aim of improving the health of people with these rare conditions.